Transcript CARDIOVASCULAR SYSTEM & AUTOIMMUNE DISEASES

CARDIOVASCULAR SYSTEM &
AUTOIMMUNE DISEASES
Praveen Kumar Shenoy
SLE
• Damage by tissue-binding autoantibodies and
immune complexes
• A multigenic disease
• More common in women and can occur at any
age.
• Diagnosis of SLE is based on characteristic clinical
features and autoantibodies.
• SLE may involve one or several organ systems;
over time, additional manifestations may occur
Cardiovascular Manifestations
• Pericarditis is the most commonly recognized
cardiac problem[30%]
• Coronary arteritis, resulting in ischemic
syndromes, rarely occur.
• In SLE, myocardial infarctions are primarily
manifestations of accelerated atherosclerosis.
• Risk factors for accelerated atherosclerosis
include disease duration, period of time treated
with corticosteroids, postmenopausal status and
hypercholesterolemia.
• Additional causes of ACS in SLE include
thrombosis, often related to the presence of
APLA, and embolism from NBTE(LibmanSacks).
• Valvular pathology in SLE is common.
• PAH can occur
• Pericardial effusion , myocarditis & arrythmias
are rare.
• Babies born to mothers with SLE and have an
increased incidence of congenital complete AV
block.
TREATMENT
• No single treatment.
• Life-threatening organ involvement →highdose corticosteroids, +/- cyclophosphamide,
azathioprine, or mycophenolate.
• Myocarditis with depressed left ventricular
function, pericarditis with impending
tamponade – an indication for cytotoxics
• Surgery
Antiphospholipid Antibody Syndrome
• Defined as the presence of either APLA or a lupus
anticoagulant and a history of otherwise
unexplained recurrent venous or arterial
thrombosis, or frequent second or third trimester
miscarriages.
• Cardiac manifestations include thrombotic CAD,
intracardiac thrombi, and NBE. Heart valve
abnormalities occur in approximately 30 percent
of patients with primary APLAS and include
leaflet thickening, thrombotic masses extending
from the valve ring or leaflets, or vegetations.
• Pulmonary hypertension can occur in patients
with APLA secondary to chronic
thromboembolic disease. APLA may promote
pulmonary artery intimal proliferation.
• Catastrophic APLA
• Revised Sapporo classification criteria – for
diagnosis
• Treatment – Anticoagulation
• In catastrophic APS other options may be
considered in desperate situations.
Systemic sclerosis
• Chronic systemic disorder of unknown
etiology.
• Early stage → prominent inflammation,
followed by widespread functional and
structural alterations in multiple vascular beds
and progressive visceral organ dysfunction
due to fibrosis.
• Mainly 2 subtypes
• Pericardial involvement is common in PSS, and
includes fibrinous pericarditis in up to 70
percent of patients at autopsy.
• The presence of moderate or large pericardial
effusions is an independent risk factor for
mortality.
• Cardiac involvement in SSc may be due to
ischemic damage, myocarditis, replacement
fibrosis, systemic hypertension, and PAH.
• Myocardial involvement may be due to
myocardial ischemia, fibrosis, and myocarditis.
• Ventricular conduction abnormalities are
common and, along with a septal pseudoinfarct pattern, correlate with reduced
myocardial function with exercise.
• Renal crisis may be associated with minimal or
extreme hypertension, rapidly rising creatinine
level, microangiopathy, thrombocytopenia,
and left ventricular failure.
• Pulmonary hypertension occurs in both
limited scleroderma and PSS
• Outcome in SSc-associated PAH is considerably
worse.
Treatment
• No therapy to alter natural history.
• Immunomodulators – only cyclophosphamide of
pronven role in RCT
• Steroid use is ideally restricted to patients with
myositis, symptomatic serositis, the early
edematous phase of the skin disease, refractory
arthritis, and tenosynovitis.
• The lowest possible therapeutically effective dose
should be used as renal crisis may be
precipitated.
• Various options for Raynaud’s & PAH.
Rheumatoid Arthritis
• Most common form of chronic inflammatory
polyarthritis
• Chronic symmetrical polyarthritis that affects
small and large joints
• Affects pericardium mostly. Chronic,
asymptomatic effusive pericardial disease is more
common
• Does not usually cause clinically significant
myocarditis but CHF seen with increased
prevelance
• Secondary amyloidosis – rare, It can cause
cardiomyopathy & AV block.
• Leading cause of death is cardiovascular
disease, with a relative risk of at least 2.
• Potential risk factors for CAD in patients with
RA - the chronic systemic inflammatory state,
generation of proatherosclerotic HDL forms,
use of selective or nonselective NSAIDs, underusage of aspirin, and use of steroids, which
may accelerate atherosclerosis.
• Coronary arteritis & valve involvement - rarely
reported
Treatment
• Disease-modifying therapy - methotrexate,
sulfasalazine, leflunomide, hydroxy-chloroquine,
and low-dose prednisolone is frequently used.
• Clinical pericarditis - use of NSAIDs, intensified
systemic immunosuppressive therapy, pericardial
steroid injections, or pericardiocentesis if
hemodynamic compromise occurs.
• Recurrent pericardial effusions may require a
pericardial window. Constriction should be
surgically treated.
• Be vigilant with newer agents in presence of CHF
Ankylosing spondylitis
• Chronic inflammatory disease of unknown
cause associated HLA-B27
• TNFα - plays a central role in the immunopathogenesis of AS.
• Features - Low back pain and stiffness,
enthesitis, chest pain, joint involvement,
uveitis, slowly progressive fibrosis of the
upper lobes of the lungs, neurological
syndromes, renal involvement & osteoporosis
• Aortic root disease- reported in up to 100
percent of AS patients who also had aortic
valve involvement in an autopsy series.
Characteristic findings - thickening of the
aortic root with subsequent dilation. Aortic
cusp nodularity with proximal thickening seen.
• Cardiac conduction disease has been well
described & more common in males
• Pericarditis & CAD rare
• Treatment – Drugs, surgery
Polymyositis and Dermatomyositis
• Localized or generalized myocardial
dysfunction is common by echocardiographic
assessment, but infrequently causes clinical
failure.
• The cardiomyopathy may be steroidresponsive.
• PM and dermatomyositis frequently affect the
conduction system.
Sarcoidosis
• Granulomatous inflammatory disease of
unknown cause.
• Pericarditis though uncommon – usually
clinically insignificant
• Granulomatous infiltrative disease of the
myocardium is often asymptomatic, but can
cause arrhythmias, conduction disease and,
rarely, otherwise unexplained congestive
heart failure
• Pulmonary artery hypertension and cor
pulmonale can occur in sarcoidosis, generally
as a result of pulmonary fibrosis.
• Systemic vasculitis - an uncommon
complication of sarcoidosis.
• Sarcoid vasculitis can affect small- to largecaliber vessels, including the aorta.
• Treatment - corticosteroids
VASCULITIS
• Heterogeneous group of disorders linked by
the primary finding of inflammation within
blood vessel walls.
• Can be primary or secondary
• Constitutional symptoms: fever, weight loss,
malaise, arthralgias/arthritis (common to
vasculitides of all vessel sizes)
Takayasu Arteritis
• The pulseless disease or occlusive
thromboaortopathy
• Most frequently in young women.
• Most commonly in Japan, China, India, and
Southeast Asia.
• Arterial stenoses 3-4 times more often than
aneurysms. Claudication (upper [60 %] versus
lower extremities [30 %]) is the most common
complaint and bruits (approximately 80 percent),
blood pressure, and pulse asymmetries (60-80 %)
are the most common findings.
• Aneurysms are most common and clinically most
significant in the aortic root, where they can lead
to valvular regurgitation (approximately 20
percent)
• Hypertension is most often caused by renal artery
stenosis, but can also be associated with
suprarenal aortic stenosis or a chronically
damaged, rigid aorta.
• Cardiac, renal, and central nervous system (CNS)
vascular diseases are the principal causes of
severe morbidity and mortality.
• Coronary artery vasculitis is rare(< 5 %), & is most
frequent in the ostial regions.
Treatment
• Corticosteroids are the cornerstone of treatment
of active TA.
• Initial dose of prednisone is continued for 4 to 12
weeks before commencing a gradual taper.
• Frequently requires revascularization procedures
• Surgical intervention should be deferred until TA
is in remission
• Bypass surgery yields better results than
angioplasty. With bypass graft procedures,
autologous vessels give better results than
synthetic grafts
Giant Cell Arteritis
• Cause of GCA remains unknown, the
inflammatory lesion begins in the adventitia.
• Most characteristic features of GCA are new
onset of atypical and often severe headaches,
scalp and temporal artery tenderness, acute
visual loss, polymyalgia rheumatica, and pain in
the muscles of mastication.
• GCA may produce clinically apparent aortitis in
~15 % of cases and involve the primary branches
of the aorta, especially the subclavian arteries,
Treatment
• Prednisone (0.7 to 1 mg/kg/day) will reduce
symptoms within 1 to 2 days and often
eliminate symptoms within 1 week. About 2 to
4 weeks after clinical and laboratory parameters, tapering of CS can begin.
Churg-Strauss Syndrome
• Rare syndrome that includes a history of asthma,
eosinophilia, pulmonary infiltrates, upper airway
inflammation, and a variable frequency of renal,
neurological, cutaneous, and cardiac
involvement.
• Cardiac disease in CSS is the most common cause
of death. It is reported in 15 to 55 percent of
cases and may include pericarditis, myocarditis,
and coronary arteritis. Congestive heart failure
occurs in 15 to 30 percent of cases.
• Corticosteroids are mainstay.
Cyclophosphamide is another option.
Polyarteritis Nodosa
• Nongranulomatous disease of only mediumsized arteries.
• Necrotizing changes seen, with weakening of
the vessel wall and aneurysm formation or
myointimal proliferation, causing stenosis and
occlusion.
• Features include
– painful nodules (similar to erythema nodosum) or
– infarction and gangrene (30 to 50 percent),
– neuropathy (especially mononeuritis multiplex, 20
to 50 percent),
– renal infarction and insufficiency (approximately
10 to 30 percent),
– hypertension (approximately 30 percent),
– segmental pulmonary infarctions (less than 40
percent), and
– cardiac disease (10 to 30 percent; congestive
failure, angina, infarction, pericarditis).
• PAN-MPA–like spectrum obligates a search for bacterial and fungal
infections as causes of endocarditis or endovascular vegetations.
Kawasaki Disease
• Acute febrile systemic illness of childhood.
• Features – fever, conjunctivitis, adenopathy, rash,
mucocutaneous changes & others
• Cardiac abnormalities - pericardial effusions (~ 30
%), myocarditis, mitral regurgitation (~ 30 %),
aortitis and aortic regurgitation (infrequent),
congestive heart failure, and atrial and ventricular
arrhythmias.
• Deaths usually result from acute coronary artery
thrombosis in aneurysms that form following
vasculitis.
Treatment
• High dosages of aspirin and IVIG
• Aspirin - 80 to 100 mg/kg/day until the patient
is afebrile After fever subsides, the dose of
aspirin is reduced (3 to 5 mg/kg/day) to
achieve primarily antiplatelet effects.
• Lifelong aspirin if aneurysms persist
Clinical
Diagnosis
Non specific
Blood tests
Imaging
Autoantibodies
Specific
Biopsy
Non specific
CBC
Acute phase
reactants
Others
ESR
Serology
CRP
RFT
LFT
Conventional
Radiography
USG
CT
IMAGING
MRI
Echocardiography
PET
Research tools
Scintigraphy
ANA
ANA profile
Autoantibodies
ANCA
Anti CCP
Diseasea
Pericardium
Myocardium
SLE
++
+
Systemic
Sclerosis
+
++
PAN
+/-
+
Ankylosing
Spondylitis
0
RA
++
Endocardium
(Valves)
Coronary
Arteries
Peripheral
Vessels
+
+
++
+
0
++
+
+/-
++
0
0
+
+
+
0
Polymyositis/ ++
dermatomyosi
tis
++
+/-
+/-
0
Takayasu
Arteritis
0
0
+
+
++
APLA
0
0
+
+
++
CSS
+
+
0
+
+/-
GCA
0
0
0
0
++
Kawasaki d/s
+
+
+/-
+/-
0
a ++0